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377545

OTC (Ornithine Carbamoyltransferase, Mitochondrial)

Size

£620.00

SKU:
377545
Application:
WB
Purity:
Purified by immunoaffinity chromatography.
Storage Conditions:
-20°C
Supplier:
United States Biological
Host:
Rabbit
Immunogen:
Synthetic peptide corresponding to aa33-70, NKVQLKGRDLLTLKNFTGEEIKYMLWLSADLKFRIKQK from human OTC at N-terminal, different from the related mouse and rat sequences by 3aa.
Format:
Supplied as a lyophilized powder from PBS, 5% BSA, 0.05% sodium azide. Reconstitute with 200ul sterile ddH2O.
Extra Details:
Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (Pi). This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may also play a role in that disease. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Western Blot: 0.1-0.5ug/ml Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized and reconstituted products are stable for 12 months after receipt at -20°C. Reconstitute with sterile dH2O. Aliquot to avoid repeated freezing and thawing. Store at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Shipping Conditions:
Blue Ice